title: Survival after lung transplantation in systemic sclerosis. A systematic review.
abstract: BACKGROUND
  Lung transplantation is a life-saving option for systemic sclerosis (SSc)-associated pulmonary arterial hypertension (PAH) and interstitial lung disease (SSc-ILD) patients. However, some programs may be concerned about the possibility of excess post-transplantation mortality related to the extra-pulmonary manifestations of SSc. The objective of this study was to evaluate survival of SSc patients post-lung transplantation. We secondarily evaluated SSc lung transplant recipient characteristics (age, sex, and type of SSc lung disease), and discussed post-lung transplantation survival of SSc patients and non-SSc patients (idiopathic PAH, and ILD).
  
  
  METHODS
  A systematic review of MEDLINE, EMBASE, Cochrane Central Registry of Controlled Trials and CINAHL (all inception to 2012) was performed to identify studies evaluating post-lung transplant survival in SSc compared to PAH and ILD patients. Two reviewers independently abstracted study and survival data.
  
  
  RESULTS
  Two hundred twenty-six citations were screened to identify 7 observational studies reporting SSc patients who underwent single lung, double lung, or heart-lung transplantation. Mean age at transplantation ranged 46-53 years. SSc post-transplantation survival ranged 69%-91% at 30-days, 69%-85% at 6-months, 59%-93% at 1-year, 49%-80% at 2-years, and 46%-79% at 3-years. Causes of death included graft failure, infection, cardiac events, hemorrhagic stroke, respiratory failure, malignancy, pulmonary hypertension, complications of bronchiolitis obliterans syndrome, anesthetic complication, and scleroderma renal crisis. There were no reports of recurrence of SSc in the lung allograft.
  
  
  CONCLUSIONS
  The short-term and intermediate-term survival post-lung transplantation are similar to IPAH and ILD patients requiring lung transplantation.
authors.affiliation: Pulmonary Hypertension Programme, University Health Network, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Irfan Y
authors.initials: IY
authors.lastname: Khan
authors.forename: Lianne G
authors.initials: LG
authors.lastname: Singer
authors.forename: Marc
authors.initials: M
authors.lastname: de Perrot
authors.forename: John T
authors.initials: JT
authors.lastname: Granton
authors.forename: Shaf
authors.initials: S
authors.lastname: Keshavjee
authors.forename: Cathy
authors.initials: C
authors.lastname: Chau
authors.forename: Amie
authors.initials: A
authors.lastname: Kron
authors.forename: Sindhu R
authors.initials: SR
authors.lastname: Johnson
mesh_terms: D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000401:mortality / Q000601:surgery*; D053208:Kaplan-Meier Estimate; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000401:mortality / Q000601:surgery*; D016040:Lung Transplantation / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality*; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000401:mortality / Q000601:surgery*
keywords: Interstitial lung disease; Lung transplantation; Pulmonary arterial hypertension; Scleroderma; Survival; Systemic sclerosis